Radiotherapy for glomus jugulare paraganglioma.

Surgery has been long considered to be the treatment of choice for glomus jugulare paragangliomas, as it is the only modality able to totally eradicate the tumour. However, despite considerable progress in interventional radiology and nerve monitoring, surgery is associated with an unacceptably high complication rate for a benign tumour, explaining the growing place of radiotherapy in the management of these tumours. This review of the literature confirms the efficacy of conformal radiotherapy with or without intensity modulation and stereotactic radiotherapy, which both achieve tumour control rates ranging from 90% to almost 100% of cases, but for different tumour volumes, almost constant stabilization or even improvement of symptoms, and a considerably lower rate of adverse effects than with surgery. However, radiotherapy remains contraindicated in the presence of intracranial invasion or extensive osteomyelitis. In the light of these results, together with the improved quality of life and a better knowledge of the natural history of this disease, many authors propose radiotherapy as first-line treatment for all glomus jugulare paragangliomas regardless of their size, particularly in patients with no preoperative deficits.

Malignant head/neck paragangliomas. Comparative study.

BACKGROUND: The objective of this study was to report 11 cases of malignant head and neck paraganglioma and to compare their epidemiological, clinical, and genetic characteristics, their natural history and their treatment with those of a series of 131 benign paragangliomas. PATIENTS AND METHODS: Retrospective analysis of 142 patients with head and neck paraganglioma managed between 2001 and 2008. Age at the time of diagnosis, gender, primary tumour site, presence of other non-head/neck paragangliomas and/or metastases diagnosed by imaging (CT, MRI, Octreoscan or (18)F-FDG PET), histology, urinary catecholamine and metanephrine levels, family history, and genetic test results were recorded. RESULTS: This series comprised 131 benign head and neck paragangliomas, mostly observed in women with a mean age at diagnosis of 45 years and a predominance of tympanojugular sites (followed by carotid and vagal sites) with 5% of secreting tumours and 20% of multifocal tumours. Eleven patients (7.7%) with a 1:1 sex ratio presented criteria of malignancy. These patients, with a lower mean age (38 years), predominantly presented carotid lesions with a higher rate of secreting and multifocal tumours, 27% and 46% respectively. The main sites of metastases were bone and lymph nodes. No tympanic paragangliomas were observed. CONCLUSIONS: Malignant paragangliomas are mainly observed in young patients with multifocal tumours, particularly carotid tumours, and are predominantly related to subunit SDH-B mutation. The work-up in these high-risk patients must include whole body scintigraphy and spine MRI. Malignancy is not necessarily associated with a poor short-term prognosis due to the slow course of the disease.

First bite syndrome.

Based on a review of the indexed medical literature (PubMed database), the authors describe the clinical features leading to the diagnosis of first bite syndrome, the pathophysiology of this syndrome and analyse the various treatment options available to otorhinolaryngologists to manage this syndrome.

Vestibular schwannoma and cell-phones. Results, limits and perspectives of clinical studies.

The widespread development of cell-phones entails novel user exposure to electromagnetic fields. Health impact is a public health issue and a source of anxiety in the population. Some clinical studies reported an association between cell and cordless phone use and vestibular schwannoma; others found none. A systematic review was performed of all published clinical studies (cohort, registry, case-control and validation studies), with analysis of results, to determine the nature of the association and the level of evidence. Cohort studies were inconclusive due to short exposure durations and poor representativeness. Registry studies showed no correlation between evolution of cell-phone use and incidence of vestibular schwannoma. Case-control studies reported contradictory results, with methodological flaws. Only a small number of subjects were included in long-term studies (>10 years), and these failed to demonstrate any indisputable causal relationship. Exposure assessment methods were debatable, and long-term assessment was lacking. An on-going prospective study should determine any major effect of electromagnetic fields; schwannoma being a rare pathology, absence of association will be difficult to prove. No clinical association has been demonstrated between cell and cordless phone use and vestibular schwannoma. Existing studies are limited by their retrospective assessment of exposure.

Success and failure factors for hearing-aid prescription: results of a French national survey.

OBJECTIVES: To identify epidemiological, socioeconomic, audiometric and environmental factors of success and failure of hearing-aid prescription, and to assess hearing-aid efficacy at 6-9 months after prescription. PATIENTS AND METHODS: A prospective nationwide survey was conducted in France on 184 patients with age-related hearing loss. Inclusion data were collected by a questionnaire filled out by the ENT specialist and patient, and with a second questionnaire filled out by telephone contact with the patient 6-9 months later. RESULTS: One-third of patients failed to fulfill the prescription, either for financial reasons or for lack of interest in correcting their disability. For the other two-thirds, the factors favoring consultation with a hearing-aid fitting specialist seemed to be: leisure activity requiring good hearing, living in a couple or family, spontaneous initial ENT consultation, strong motivation, monthly income greater than €1200, longstanding hearing impairment, and difficulty in listening to television and following a conversation in noise. Eighty percent of hearing-aid trials were successful; 60% of prescriptions were thus followed by hearing-aid purchase. The main three criteria determining purchase were the advice of the hearing-aid fitting specialist, and the price and the effectiveness of the apparatus on trial. In the four daily life situations presented in the questionnaire, the hearing-aid was worn for 8 hours or more in 90% of cases, found useful in 70% and proved satisfactory in 70%. Age-related hearing loss, whether metabolic or sensorineural, benefited from hearing-aid correction in 86% of cases. CONCLUSIONS: Indications for hearing-aid prescription should take account of the patient's degree of motivation, awareness of disability, and income. The advice of the ENT and hearing-aid fitting specialists plays a key role in the patient's acceptance of the hearing-aid. Hearing-aids seem to enhance quality of life significantly in age-related hearing loss subjects.

First Bite Syndrome: a little known complication of upper cervical surgery.

INTRODUCTION: First Bite Syndrome (FBS) is a rare pain syndrome sometimes occurring after surgery of the upper cervical region. It presents as excruciating pain, triggered at the beginning of a meal by chewing, swallowing or even simple contact with generally acidic food, waning on subsequent bites and recurring with identical features after pausing for several minutes or at the next meal. OBJECTIVES: Retrospective review of 17 patients who developed FBS after upper cervical surgery. RESULTS: Seventeen patients developed FBS between 1999 and 2010 following surgery for paraganglioma in eight cases, vagal or sympathetic schwannoma in five cases (including one malignant tumour), pleiomorphic adenoma in three cases and Warthin's tumour of the deep lobe of the parotid in one case. The cervical sympathetic trunk was sacrificed in 10 cases and the external carotid artery was ligated in six cases. Horner's sign was observed postoperatively in 12 patients. The characteristic pain of FBS was triggered by chewing or simple contact with essentially acidic food. CONCLUSION: FBS must be identified by the head and neck surgeon and distinguished from the usual postoperative pain. The generally accepted hypothesis is that of sympathetic denervation with parasympathetic secretory hyperactivity, but Horner's sign was present in only 12 of the 17 patients of our series, suggesting that other pathogenic mechanisms may be involved. FBS is difficult to treat, but the pain gradually becomes less severe. The patient must be informed about this rare complication that can impact on postoperative quality of life.

Jugular foramen paragangliomas: management, outcome and avoidance of complications in a series of 75 cases.

Jugular foramen paragangliomas are rare skull base tumours posing multiple complex diagnostic and management problems. We did a study to evaluate surgical technique, outcome and complications in 75 cases of tumours treated by multidisciplinary approach (i.e. combined neurosurgery, neuroradiology, ear, nose and throat surgery and intensive care unit team). Retrospective study on 75 consecutive patients with jugular foramen paragangliomas treated surgically from 1989 to 2005. Preoperative balloon occlusion test was performed in all patients as well as embolization (100%). A combined limited infratemporal and juxtacondylar approach was used in all patients. Gross total resection was achieved in 59 patients (78.7%). The most common complication was represented by lower cranial nerve deficits in five patients (6.6%), which was only temporary in three. Postoperative facial nerve weakness occurred in five cases (6.6%) and resolved in three of them. The remaining two patients underwent facial nerve reconstruction by hypoglossal/facial nerve anastomosis. Four patients (5.3%) had a postoperative cerebrospinal fluid leak, which was successfully treated by lumbar drainage. Two patients (2.7%) died because of complications related to surgical injury of lower cranial nerves: one patient developed aspiration pneumonia and septicemia and the second one developed a large cervico-bulbar hematoma that led to severe respiratory distress and ultimately global cerebral hypoxia. Paragangliomas are rare and complex skull base lesions that may be managed with low morbidity and mortality if a multidisciplinary approach is considered. Facial and lower cranial nerve postoperative deficits can be limited.

[The ultra-rapid cinematography of the larynx, its contributions in speech pathology]. / La cinématographie ultra rapide du larynx, ses apports en phoniatrie.

The development in the medical field of high speed cinematography and its dissemination in the field of speech pathology will probably change the way we view the larynx and diagnose its disorders. So far only the stroboscope could inform us about the appearance of vocal cord vibration but with certain limitations. Indeed the wave motion of the vocal cords is really only apparent motion. It is made possible through the phenomenon of retinal persistence of images and light intermittent vocal folds set to the frequency of the voice and out of phase by a few Hertz. This technique has several disadvantages: The need for the voice to trigger the strobe light; a low number of frames per second (25 fps) recorded; frame loss for the period between unlit two flashes; limitation in the study of the upper voice spectrum (gearing). The ultra-rapid cinematography brings a big difference in design since the digital recording can shoot up to 4000 frames per second with permanent lighting of the larynx. The slow reading of short sequences permits us to view the actual movement of vibrating structures, and to analyze the behavior of the vibrator during the transitional phases of the attack, depreciation and termination of sound. The footage in high resolution permits a detailed analysis of the movements of opening and closing of the vocal cords in phonation and respiration, and the diagnosis of lesions.

Writing an article for the European Annals of Otorhinolaryngology, Head and Neck Surgery: rights and responsibilities.

Based on a review of the literature published on medical writing, the authors discuss the rules to respect in terms of both structure and substance to facilitate publication of studies in the European Annals of Otorhinolaryngology, Head and Neck Surgery. The main errors leading to an article's being rejected are detailed and analyzed.

Isolate vertigo crisis revealing an endolymphatic sac tumor.

UNLABELLED: Endolymphatic sac tumors are rare entities that have a destructive potential on the temporal bone. They are aggressive tumors presenting as low-grade papillary adenocarcinoma, but there are no reports of metastasis in the literature. The Von Hippel-Lindau disease is a hereditary condition caused by germinal mutations of the tumor-suppressor VHL-gene. We present the case of an endolymphatic sac tumor associated with the Von Hippel-Lindau disease at a 46-year-old patient revealed by an isolate vertigo crisis, discussing the management of the tumor and the clinical, imaging, genetic and histopathologic features of it. CONCLUSIONS: Endolymphatic sac tumors have recently been described as part of the Von Hippel-Lindau disease, a genetic disorder involving the development of hypervascular tumors. The treatment depends on the size of the tumor, however surgical approach is the most successful choice and can be associated or not with radiotherapy.

Eustachian tube melanoma treated by double surgical approach.

OBJECTIVES: We report a case of an eustachian tube melanoma treated by a double surgical approach. MATERIAL AND METHODS: A 56-year-old man consulted for a recurrent left serous otitis media. RESULTS: CT-scan and MRI showed a lesion filling completely the middle ear, extending along the Eustachian tube up to the nasopharynx, with enhancement after injection. The biopsy of the lesion confirmed the diagnosis of malignant melanoma. After left internal carotid artery occlusion, the removal of the lesion was performed by a double surgical approach: combination of an endoscopic transnasal and a retroauricular approach. Radiotherapy was adjuvant therapy. CONCLUSION: Eustachian tube melanoma is a rare occurrence. Surgical treatment must be radical, what represents a challenge considering the localization; it can be obtained with the double surgical approach proposed in our case report.

[Association of an arachnoid cyst with heterotopic neuroglial tissue in the internal auditory canal]. / Association d'un kyste arachnoïdien et d'une hétérotopie de tissu glial dans le méat auditif interne.

OBJECTIVES: We report a case of an association of an arachnoid cyst with heterotopic neuroglial tissue in the internal auditory canal. MATERIAL AND METHODS: A 66-year-old woman consulted for cochleovestibular syndrome. RESULTS: MRI demonstrated a lesion with spontaneous hypersignal on T1- and T2-weighted images, instigating surgical exploration. We discovered a hematic arachnoid cyst associated with heterotopic neuroglial tissue arising in the internal auditory canal. CONCLUSION: An arachnoid cyst arising within the cerebellopontine angle or the internal auditory canal is a rare occurrence. Clinical manifestations are identical with those produced by a cochleovestibular schwannoma. MRI usually demonstrates a nonenhancing isointense cystic mass with cerebrospinal fluid on all sequences (hypointense on T1-weighted and hyperintense on T2-weighted images). These lesions are usually monitored. Spontaneous hypersignal on T1- and T2-weighted images makes diagnosis difficult, as in our case, leading to surgical exploration.

Role of vestibular input in triggering and modulating postural responses in unilateral and bilateral vestibular loss patients.

The aim of this study was to determine whether the greater medial-lateral (ML) instability observed in patients with compensated unilateral vestibular loss (UVL), tested on a seesaw platform with eyes closed, is task-dependent. UVL patients, categorized into three groups according to time since lesion (1 week, 1 month and 1 year), bilateral vestibular loss patients and age-matched healthy control subjects were tested in three dynamic postural tasks. These tasks involved different supports - a seesaw platform (Satel), a platform generating horizontal linear translations (Synapsys) and foam rubber placed on a static platform - each requiring different somatosensory cues to maintain equilibrium. Displacements of the subjects' center of pressure in both the anterior-posterior (AP) and ML directions were recorded by strain gauges within the platforms. Only tests performed with eyes closed were analyzed. Bilateral vestibular loss patients fell during foam and seesaw trials but not on the platform generating translations. We previously reported that UVL patients had greater postural oscillations on the seesaw platform in the ML compared to AP direction. In this study, we show similar ML/AP differences in patient performance on foam when standing with 'feet close together'. In contrast, these differences were not found when patients were tested on linear translation or on foam standing with feet apart. In conclusion, the postural performance of patients with vestibular loss depends on the exact task used to measure postural stability. UVL patients are less stable when subjected to movement in the ML direction because of the biomechanical constraints of the tasks and/or the availability of proprioceptive information.

[Dilation of the internal auditory canal and intracanalicular vestibular schwannoma: what are the mechanisms involved?]. / Dilatation du méat acoustique interne et schwannome vestibulaire intracanalaire: quels mécanismes?

OBJECTIVES: To present a unique case of unilateral widening of the internal auditory canal (IAC) with no significant contact with an ipsilateral intracanalicular vestibular schwannoma (VS), raising the issue of the cause(s) of this IAC widening. METHODS: The medical record and radiologic data were reviewed of a patient presenting an enlarged unilateral IAC, which led to the diagnosis of an intracanalicular VS that could not account for the dilation. RESULTS: The patient had a unilateral dilation of the IAC that did not match the ipsilateral VS he had. As a result, this case motivated discussion of whether such dilation of the IAC was congenitally asymmetrical or the result of the mechanisms involved in the widening of the IAC. CONCLUSIONS: Although asymmetry of IAC is a current notion, this case demonstrates a contrario that increased pressure exerted on the walls of the IAC cannot be the only mechanism in such widening.

Spectrum of skull base tumors in children and adolescents: a series of 42 patients and review of the literature.

OBJECTIVE: This study aims to contribute to the collective experience with the treatment of those skull base tumors exceptionally arising in children and adolescents. MATERIALS AND METHODS: We retrospectively reviewed 42 patients younger than 19 years and operated on for a skull base tumor between 1992 and 2002. We analyzed data regarding patient age, histology, tumor location, extent of resection, postoperative complications, and adjuvant therapies. Long-term outcomes were also tracked (functional sequelae, recurrence, mortality). CONCLUSION: The median age was 13.75 years. Twenty-two patients harbored a malignant tumor. Gross total resection was achieved for 78% of cases. Transient postoperative cerebrospinal fluid leak or infection affected ten patients, suggesting the need for reconstructive techniques. Nineteen patients received adjuvant therapy. Recurrence rate was 47%. At the end of the follow-up (median = 63 months), 14% of patients had a neurological deficit and 30% were dead. Middle cranial base tumors were associated with a poor prognosis.